| Experiment Id | GSE72151 | Name | Transcriptome analysis of Largemyd and Dmdmdx/Largemyd muscles in comparison to Dmdmdx: what make them different? |
| Experiment Type | transcription profiling by array | Study Type | WT vs. Mutant |
| Source | GEO | Curation Date | 2024-04-11 |
| description | Transcriptome analysis of hindlimb muscles from dystrophic mice Muscular dystrophies (MD) are a clinically and genetically heterogeneous group of mendelian diseases. The underlying pathophysiology and phenotypic variability in each form are much more complex, suggesting the involvement of many other genes. Thus, here we studied the whole genome expression profile in muscles from three mice models for MD, at different time points: Dmdmdx, carrying a mutation in dystrophin gene, Largemyd-/- with mutation in Large and Dmdmdx/Largemyd-/- bearing both mutations. The main objective was to identify altered biological functions, contributing to the understanding of disease and to the identification of prognostic biomarkers and points for therapeutic intervention. We identified a substantial number of differentially expressed genes (DEGs) in each model, reflecting diseases' complexity. The main biological process affected in the three strains was immune system, accounting for the majority of enriched functional categories, followed by degeneration/regeneration and extracellular matrix remodeling processes.The most notable differences were in 21-day-old Dmdmdx, with a high proportion of DEGs related to regenerative capacity observed in this mouse. The new Dmdmdx/Largemyd-/- model did not show a highly different transcriptome from the parental lineages, with a profile closer to Largemyd-/-, but not bearing the same regenerative potential as Dmdmdx. This is the first report about transcriptome profile of a mouse model for congenital MD and Dmdmdx/Largemyd. By comparing the profiles studied, we conclude that alterations in biological functions due to the dystrophic process are very similar, and that the intense regeneration in Dmdmdx involves a large number of activated genes, not differentially expressed in the other two strains. We analyzed calf muscles from 5 animals of each lineage (Dmdmdx, Largemyd, Dmdmdx/Largemyd and C57Bl/6 as normal control), at three ages (21 days, 3 months and 6 months) using the Affymetrix Mouse Gene 1.0 ST platform. Array data was processed by Expression Console. No technical replicates were performed. |
